Recurrent unilateral pneumonia poses a diagnostic dilemma for all paediatricians. Isolated pulmonary venous atresia is an uncommon entity, which presents with recurrent unilateral pulmonary infections, dyspnoea on exertion and haemoptysis. It is associated with significant mortality and morbidity. Owing to rarity of condition, there may be some difficulties in the diagnosis and therapy. Pulmonary angiography is the investigation of choice. However, the current multislice CT scanners may obviate the need for pulmonary angiography. We report a two-year-old child with this rare congenital malformation and discuss diagnostic modalities and management options.